McFadden Jason R, Chaudhari Advaita S, Birenbaum Debra, Margesson Lynette, Gonzalez Jorge, Sriharan Aravindhan
Department of Biological Sciences, Dartmouth College, Hanover, NH 03755, USA.
Department of Pathology & Laboratory Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH 03766, USA.
Dermatopathology (Basel). 2023 Feb 2;10(1):63-69. doi: 10.3390/dermatopathology10010009.
Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is a heterogeneous group of subepidermal blistering diseases that affect the mucous membranes, most frequently in the eye and oral cavity. MMP is often unrecognized or misdiagnosed in its early stages due to its rarity and nonspecific presentation. We present the case of a 69-year-old female in which MMP of the vulva was not initially suspected. The first biopsy, from lesional tissue for routine histology, revealed fibrosis, late-stage granulation tissue, and nonspecific findings. A second biopsy, from perilesional tissue for direct immunofluorescence (DIF), revealed DIF findings typical of MMP. Scrutiny of both the first and second biopsies revealed a subtle but telling histologic feature: subepithelial clefts along adnexae in the context of a scarring process with neutrophils and eosinophils, which can be an important clue to MMP. This histologic clue has been previously described; reinforcing its importance may prove useful for future cases, especially those for which DIF is not feasible. Our case demonstrates the protean presentations of MMP, the need for persistence in sampling unusual cases, and the relevance of inconspicuous histologic features. The report highlights this underrecognized yet potentially decisive histologic clue to MMP, reviews current biopsy guidelines when MMP is suspected, and delineates the clinical and morphological features of vulvar MMP.
黏膜类天疱疮（MMP），也称为瘢痕性类天疱疮（CP），是一组异质性的表皮下疱性疾病，累及黏膜，最常见于眼和口腔。由于其罕见性和非特异性表现，MMP在早期常未被识别或误诊。我们报告一例69岁女性病例，最初未怀疑其患有外阴MMP。首次活检取自病变组织进行常规组织学检查，结果显示纤维化、晚期肉芽组织及非特异性表现。第二次活检取自病变周围组织进行直接免疫荧光（DIF）检查，结果显示为MMP典型的DIF表现。对首次和第二次活检结果的仔细检查发现了一个细微但有提示意义的组织学特征：在伴有中性粒细胞和嗜酸性粒细胞的瘢痕形成过程中，附件周围的上皮下裂隙，这可能是MMP的一个重要线索。这一组织学线索此前已有描述；强调其重要性可能对未来病例有用，尤其是那些无法进行DIF检查的病例。我们的病例展示了MMP的多种表现形式、对不常见病例持续取样的必要性以及不明显组织学特征的相关性。该报告强调了这一尚未得到充分认识但可能具有决定性意义的MMP组织学线索，回顾了怀疑MMP时当前的活检指南，并阐述了外阴MMP的临床和形态学特征。
